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Mechanism-based corrector combination restores ΔF508-CFTR folding and function.
Okiyoneda T, Veit G, Dekkers JF, Bagdany M, Soya N, Xu H, Roldan A, Verkman AS, Kurth M, Simon A, Hegedus T, Beekman JM, Lukacs GL. Okiyoneda T, et al. Among authors: xu h. Nat Chem Biol. 2013 Jul;9(7):444-54. doi: 10.1038/nchembio.1253. Epub 2013 May 12. Nat Chem Biol. 2013. PMID: 23666117 Free PMC article.
Rattlesnake Phospholipase A2 Increases CFTR-Chloride Channel Current and Corrects ∆F508CFTR Dysfunction: Impact in Cystic Fibrosis.
Faure G, Bakouh N, Lourdel S, Odolczyk N, Premchandar A, Servel N, Hatton A, Ostrowski MK, Xu H, Saul FA, Moquereau C, Bitam S, Pranke I, Planelles G, Teulon J, Herrmann H, Roldan A, Zielenkiewicz P, Dadlez M, Lukacs GL, Sermet-Gaudelus I, Ollero M, Corringer PJ, Edelman A. Faure G, et al. Among authors: xu h. J Mol Biol. 2016 Jul 17;428(14):2898-915. doi: 10.1016/j.jmb.2016.05.016. Epub 2016 May 27. J Mol Biol. 2016. PMID: 27241308
Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell.
Bagdany M, Veit G, Fukuda R, Avramescu RG, Okiyoneda T, Baaklini I, Singh J, Sovak G, Xu H, Apaja PM, Sattin S, Beitel LK, Roldan A, Colombo G, Balch W, Young JC, Lukacs GL. Bagdany M, et al. Among authors: xu h. Nat Commun. 2017 Aug 30;8(1):398. doi: 10.1038/s41467-017-00444-4. Nat Commun. 2017. PMID: 28855508 Free PMC article.
Mutation-specific downregulation of CFTR2 variants by gating potentiators.
Avramescu RG, Kai Y, Xu H, Bidaud-Meynard A, Schnúr A, Frenkiel S, Matouk E, Veit G, Lukacs GL. Avramescu RG, et al. Among authors: xu h. Hum Mol Genet. 2017 Dec 15;26(24):4873-4885. doi: 10.1093/hmg/ddx367. Hum Mol Genet. 2017. PMID: 29040544 Free PMC article.
Chaperone-Independent Peripheral Quality Control of CFTR by RFFL E3 Ligase.
Okiyoneda T, Veit G, Sakai R, Aki M, Fujihara T, Higashi M, Susuki-Miyata S, Miyata M, Fukuda N, Yoshida A, Xu H, Apaja PM, Lukacs GL. Okiyoneda T, et al. Among authors: xu h. Dev Cell. 2018 Mar 26;44(6):694-708.e7. doi: 10.1016/j.devcel.2018.02.001. Epub 2018 Mar 1. Dev Cell. 2018. PMID: 29503157 Free PMC article.
Structure-guided combination therapy to potently improve the function of mutant CFTRs.
Veit G, Xu H, Dreano E, Avramescu RG, Bagdany M, Beitel LK, Roldan A, Hancock MA, Lay C, Li W, Morin K, Gao S, Mak PA, Ainscow E, Orth AP, McNamara P, Edelman A, Frenkiel S, Matouk E, Sermet-Gaudelus I, Barnes WG, Lukacs GL. Veit G, et al. Among authors: xu h. Nat Med. 2018 Nov;24(11):1732-1742. doi: 10.1038/s41591-018-0200-x. Epub 2018 Oct 8. Nat Med. 2018. PMID: 30297908 Free PMC article.
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